ஜர்னல் ஆஃப் கிளினிக்கல் கேஸ் ரிப்போர்ட்ஸ்

Transient osteoporosis of the hip (TOH) is a condition that can cause severe and debilitating joint pain. It can be misdiagnosed as avascular necrosis of the hip (AVN). Hyperbaric oxygen therapy (HBOT) was given to a patient with severe hip pain and diagnosed with early AVN with the hopes of avoiding surgical intervention. Within 2 months, the clinical symptoms of hip pain completely resolved, and the diagnosis was changed to TOH. HBOT successfully reversed TOH, minimized the period of debilitation in this disorder and may hold promise in early AVN patients.

Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive disease caused by resistant of ACTH receptor at adrenal cortex leading to (usually) isolated glucocorticoid deficiency with normal mineralocorticoid secrete. Patients with FGD usually presented in neonatal–childhood period with signs/symptoms of glucocorticoid deficiency such as hypoglycemia, hyperpigmentation, Failure to thrive, shock and death if treatment was delayed. Labs usually revealed high ACTH, low cortisol but normal 17 OHP, electrolyte, androgen. Here we describe a 3-yearsold Saudi girl, with history of progressive hyperpigmentation for first year of life, but no history of hypoglycaemia or neonatal jaundice, no history of a lacrimation or dysphagia and positive similar family history. She had generalized Hyperpigmentation with normal female genitalia. Her cortisol was low with high ACTH level, but normal electrolyte 17, Hydroxyprogesterone, aldosterone, renin, androgen. Familial Glucocorticoid Deficiency was diagnosed, and maintenance dose of hydro cortisol was started, and patient pigmentation was improved few week latter.

Introduction: The inflammatory response to an infectious trigger may evolve to sepsis and septic shock most frequently at the extremes of age and in immunosuppressed patients. Klebsiella pneumoniae is a gram-negative bacillus that is part of the saprophytic flora of the digestive tract. Bacteremia with commensal germs may be secondary to a localized infectious event from where bacteria crosses vascular endothelium.

Case presentation: We are presenting the case of a 17-years-old patient, admitted to the National Institute of Infectious Diseases “Prof. Dr. Matei Bals” in June 2017, on the 4th day of illness. Symptoms started 8 hours after meal with nausea, vomiting, fever, and temporo-spatial disorientation. Clinical manifestations and paraclinical investigations have established the diagnosis of sepsis with Klebsiella pneumoniae.

Results: Although antibiotic treatment was promptly initiated according to the antibiogram, complications of bacteremia still occurred, and patient developed renal abscess. Evolution was favorable under antibiotic treatment and correction of modified biological parameters, the patient being discharged cured after 21 days.

Discussion: The particularity of the case is given by the immunocompetent profile of the patient who developed sepsis with commensal germ. Intestinal inflammation secondary to a food poisoning has facilitated bacterial translocation. Bacterial lipopolysaccharide (LPS) is transported by binding proteins to CD14 receptor from the effector cells (neutrophils, monocytes, macrophages). This receptor facilitates the interaction between LPS and TLR 4. As a result, synthesis of proinflammatory factors: cytokines, chemokines, prostaglandins are stimulated.

Conclusion: Klebsiella pneumoniae causes an increased level of endotoxemia. Consequently, the risk of sepsis is higher even in immunocompetent patients.

Infective endocarditis is a disease with a high mortality rate; mortality increases with the number of new episodes. Patients with relapsing endocarditis have a higher mortality rate per year (20%) than patients with a single episode (9%); moreover, the episodes of infective endocarditis on the prosthetic valve have a worse prognosis, with a mortality rate of 45% for each episode. In relapsing endocarditis, the following are distinguished: the relapses, characterized by the same germ, less than six months after the previous episode, and the reinfections, which may be caused by a different germ, or by the same germ, more than six months after previous episode. A recent analysis of the risk factors of recurrent bacterial endocarditis showed that the use of intravenous drugs, hemodialysis, immunosuppression and previous episodes of infective endocarditis played a major role, especially if they occurred on the prosthetic valve and if caused by S. aureus. Recurrent infectious endocarditis occurs more frequently in young male patients with HIV seropositivity, who routinely use intravenous drugs. We have analyzed the clinical case of a 36-year-old immunocompetent man with a long history of parenteral drug abuse, who has presented 6 new episodes of relapsing infective endocarditis on mitral valve and 2 reinfections since 2013. Between 2013 and 2016, the patient underwent two mitral valve replacements with a biological prosthesis implant and a mitral valve replacement with a mechanical prosthesis implant and a tricuspid annuloplasty. The remaining episodes were treated with medical therapy only. Currently in the literature 6 episodes of relapsing infective endocarditis and 1 reinfection in a single patient is the highest number of reported events.

Van Der knaap syndrome is a very uncommon disease seen mainly in the Aggarwal community in northern India. The characteristics of this disease are early onset macrocephaly with mild motor developmental delay, progressive gait alterations, muscular stiffness (spasticity), progressive ataxia, sporadic seizures and usually mild cognitive impairment (MCI) which is of late appearance. Only a few cases of this pathology have been reported in worldwide literature. for the knowledge of all, there are no reports so far of two siblings of parents in which there is apparently no Indian descent or consanguinity. In this case the brothers started with abnormal growth of the cephalic perimeter, difficulty to start the march which progressively got worse, muscular stiffness was developed while intellectual functioning was preserved for several years after onset of the disorder. Was more notorious in one of the two brothers in terms of motor and speech deficit.

Ameloblastoma previously known as adamantinoma is a histologically benign, locally invasive odontogenic neoplasm of epithelial origin which is derived from the odontogenic ectoderm. It was first described by Cusack in 1827. However, the first detailed description of this lesion was given by Falkson in 1879. The term ‘ameloblastoma’ was coined by Churchill in 1933. Ameloblastoma is an odontogenic tumor representing 1% of all tumors of the jaw, with 80% to 85% occurring in the mandible and 15% to 20% in the maxilla. In the maxilla, 47% of ameloblastomas have been reported in the molar region, 15% in the maxillary antrum and floor of the nose, 9% in the premolar region, 9% in the canine and incisor region and 2% in the palate. Maxillary ameloblastoma is most commonly associated with painless swelling, loosening of teeth, nasal airway obstruction, malocclusion, periodontal diseases and ulceration. The proximity of maxilla to the orbit, skull base and intracranial contents contributes to the high morbidity and mortality rate associated with them.

Objective: Deep venous thrombosis frequently occurs in patients with neoplastic diseases. The risk is increased by approximately seven times in patients receiving chemotherapy or radiation treatment. Upper extremity deep venous thrombosis is uncommon, mainly due to central venous catheters. Thymus gland tumors are rare, representing up to 1% of all primitive tumors. Adjuvant radiotherapy is recommended in presence of residual tumor after surgery.

Case Report: We report the case of a patient suffering from thymoma treated with surgery and radiotherapy, who showed echo graphic evidence of hyperechoic, endoluminal, floating formation in the innominate vein, suggestive of thrombosis that was not responsive to anticoagulant therapy.

Discussion and Conclusion: This case confirms the difficulty in diagnosing some endoluminal free-floating formations in vascular bed. Recourse to the ex adiuvantibus criterion, consisting in this case in lack of benefits with anticoagulant treatment, should be desirable.

Biliary cystadenoma (BCA) is a rare tumor which is difficult to diagnose before surgery. We present a case of 73 years old woman with a previous histological diagnosis of biliary cystadenoma occurred in 2009, surgically removed. In January 2018, abdomen Computerized Tomography scan (CTs) showed a 65 mm diameter cystic mass in the fifth hepatic segment. Even if the patient refused to undergo surgical resection due to her several comorbilities, imaging features and clinical history are suggestive for BCA recurrence.

Purpose: Despite the advantages of endovascular procedures (EVAR) some authors are doubts to recommend this method for some patients. It main concerns the patients with accessory renal arteries (ARAs) and an increased risk of kidney failure. The aim of this work was to evaluate the impact of accessory renal arteries on the renal complications after EVAR.
Methods: 54 of non-randomized patients with ARAs were enrolled to project. The endovascular aneurysm repair (EVAR) was performed in 30 patients and in other 24 classical surgery. 70 patients without ARAs composed the control. Kidney condition was estimated on RIFLE-AKIN classification evaluating concentration of creatinine, urea, glomerular filtration rate (GFR), and urine output per hour (UO). The multivariate regression analysis (ANCOVA test) was applied to correct for confounding and increase of precision of an estimated differences.
Results: The parameters after EVAR procedure did not differ significantly in comparison with preoperative value of urea (p=0.6), creatinine (p=0.16), GFR (p=0.4) and urine output (p=0.8). The ANOVA test revealed also that postoperative parameters in all group of patients did not differ significantly. Although, data in classical approach both with or without ARAs shown a slightly tendency towards renal failure by higher concentration of urea (46.1 vs. 45.1) and creatinine (1.26 vs. 1.21) and lower GFR (61 vs. 66) and urine output per hour (0.60 vs. 0.61). In multivariate regression analysis was shown the preoperative renal failure as an independent factor of acute renal injury. According to RIFLE-AKIN algorithm none parameters did not reach the expected postoperative value which allow to diagnose one of the stadia of acute kidney injury.
Conclusions: Based on the results in patients with ARAs but normal function of kidney the EVAR procedure can be considered as the safe method.

The knowledge for molecular mechanisms of preterm labor and preeclampsia is still premature. In the previous review, the author proposed that sex steroid treatment should be conserved for prospective clinical study in both preterm labor and preeclampsia. Unfortunately, little attention has been paid so far on the review article. Here, the authors present two cases of severe, complicated preeclampsia associated with preterm labor.

Psychogenic Nonepileptic Seizures (PNES) are defined as typical seizure-like activities or behaviors without cortical epileptiform discharges. The diagnosis of PNES remains a challenge and continuous video electroencephalography (cEEG) remains the gold standard to differentiate between epileptic seizures and PNES. Despite advances made in our understanding of PNES, diagnosing and treating this well-known entity remains a challenge. 25% of patients with seizures will be misdiagnosed as having PNES or epileptic seizures even by certified Neurologists. Do patient characteristics and demographics increase our accuracy to diagnose PNES? We present a case of a mother and her son, both diagnosed with PNES by cEEG monitoring in our epilepsy monitoring unit. While familial prevalence of conversion disorders is reported in the literature, this will be the first case report describing the incidence of PNES in two members of the same family. Do psychogenic epilepsies run in families? Will the diagnosis of PNES in one family member increase the probability of PNES in their relatives with seizures? Larger patient pools need to be studied to draw a definite conclusion regarding our observation.

Men Breast Cancer (MBC) is uncommon and occurs after the age of 60 years. Among men Prognosis is poor as Prognosis a course of a medical condition is discovered at a late stage. where Infiltrating ductal carcinoma accounts for (70-90%) of male breast cancers. In situ but not invasive carcinoma is exclusively ductal, and it accounts only 7% of the breast cancer cases in males. It is observed that 50-75% of breast cancer cases are spread to lymph nodes. The etiology of male breast cancer is unknown. An excess risk has been associated with the testicular disorders, benign breast disease including gynecomastia, Klinefelter syndrome, etc., Preliminary evidence suggests that BRCA2 is a strong cause. The carriers of the males with BRCA2 mutation have an increased lifetime risk of breast cancer with 80-fold in it. It is also known that there is also a risk of breast cancer associated with undescended testes and it is also related to orchiectomy, orchitis, testicular injury, etc., with an increasing number of children the decreasing trend in risk was observed gradually. Where It is also known that Liver cirrhosis is associated with increased levels of estrogens possibly via high levels of endogenous estrogens.

Torpedo maculopathy is a rare, congenital anomaly of the retinal pigment epithelium (RPE) which is characterized by the appearance of a ‘torpedo‐shaped’ lesion located temporal to the fovea. In our case a 9 years old girl on her routine eye examination was discover to have a bilateral macular RPE defect with a pointed-oval shape directed toward the foveola and hyperpigmented. OCT and Foto fundus was done to the patient and diagnoses of torpedo maculopathy was made. Regular was recommended.

We report a case of full term abdominal pregnancy with healthy newborn. This is a rare variety of ectopics pregnancies whose evolution to the end remains exceptional. It is unique to developing countries with a high incidence of genital infections and poor pregnancy monitoring. It is associated with significant perinatal mortality because of fetal malformations and maternal morbidity related to hemorrhagic, infectious and occlusive risk. Ultrasound is almost unavoidable for positive diagnosis supplemented by magnetic resonance imaging detecting fetal malformations and allowing a better analysis of placental relations with the abdominal organs; decisive elements to optimize the care of the parturient.

Introduction: Gram negative bacterial meningitis is a rare initial presentation of a macroprolactinoma. Meningitis in these cases is likely caused by a defect in the sellar floor from surgical manipulation or treatment with a dopamine agonist causing rapid shrinkage of a tumor. Patients generally have headaches as the predominant symptom. Our case is an illustration of where meningitis is caused by a transsphenoidal surgery and where meningitis is the presenting symptom to diagnosing a macroprolactinoma.
Case presentation: The patient is a 20-year old African female with history of transsphenoidal surgery over one year ago, who presents with headache associated with subjective fevers, nausea, and vomiting. The patient states that she had similar headaches for the past 2 years but not to this severity. She reports that when she was in her country one year ago she had an unknown surgery through her nose that helped the headaches for a short period of time. She recalls having increasingly irregular periods over the past year and milky discharge from her breasts.
Results: Physical exam was significant for nuchal rigidity. CT scan showed large sellar mass and MRI was consistent with likely pituitary adenoma. The patient’s presentation was suspicious for meningitis, so a lumbar puncture was performed, and CSF analysis was consistent with bacterial meningitis. The patient was started on broad spectrum antibiotics until CSF cultures returned showing Klebsiella pneumoniae sensitive to ceftriaxone. Further testing showed very high prolactin levels. Endocrinology was consulted and agreed the tumor was a pituitary macroadenoma, most likely a prolactinoma due to her laboratory findings and symptoms of menstrual irregularity and galactorrhea. The patient was treated with Cabergoline 0.5 mg two times per week.
Conclusion: Bacterial meningitis, especially when caused by gram negative organisms is a rare presentation of a pituitary macroprolactinoma. Thorough history taking, and prompt diagnostic evaluation is crucial, especially when medical records cannot be obtained. Treatment of both conditions quickly and simultaneously is important. The case also illustrates that how treating a prolactinoma can result in complications such as meningitis not just immediately after surgery but in any stage of treatment.

Liposarcoma, a type of soft tissue sarcoma, represents a therapeutic challenge, in particular in the metastatic setting. This case describes a patient with retroperitoneal liposarcoma that was first treated by surgery and, upon manifestation of metastatic disease, with a series of chemotherapy regimens comprising doxorubicin (line 1), doxorubicin plus ifosfamide (line 2), trabectedin (lines 3 and 4) and dacarbazine (line 5). While transient partial remission was achieved in the first three lines, rapid disease progression was observed in subsequent therapies. After failure of the previous chemotherapy regimens, eribulin was administered (line 6), and partial remission was achieved within five months. However, the occurrence of grade 3 polyneuropathy led to temporary discontinuation of therapy. Overall, metastatic liposarcoma was controlled for twelve months with eribulin treatment in a heavily pre-treated patient. This case underscores the efficacy of eribulin in treatment-refractory advanced/metastatic liposarcoma.